This 6-year-old girl's history was notable for complex congenital heart disease, including mild aortic valvular stenosis with postductal coarctation and secundum ASD. Moderate LVH and mild RVH were present at the time of clinical presentation. The patient developed frequent sometimes syncopal episodes of rapid SVT at exertion and failed metoprolol. When the patient entered the hospital for planned coarctation repair, concomitant EP study and surgical ablation were proposed. A left posterolateral concealed accessory pathway was discovered which was serendipitously quite easily ablated via a trans-ASD approach.

Complex Congenital Heart Disease and Arrhythmias, Part 2

Left lateral view of the same patient's heart showing site of successful ablation. Two RF applications eliminated early atrial activation at the left posterior site. After EP study, the patient proceeded to planned surgeries. Intraoperative TEE confirmed two discrete contiguous lesions on the LA side of the mitral annulus, with no adherent thrombus.

Coarctation Repair and Vascular Reconstruction

In addition to severe coarctation, the aortic valve was small, trileaflet but with mild stenotic gradient. Multiple large-caliber collaterals were present in both the posterior intercostal system and the internal mammaries. The eccentric nature of the coarctation, and significant poststenotic dilatation in the thoracic aorta, prevented the optimal placement of a graft. The wide left subclavian was taken down and applied as a patch to widen the coarct. The distal subclavian artery was reconnected to the proximal third of the left carotid via a short graft with oblique end-to-side anastomosis (not shown). On TEE, the aortic valvular gradient remained minimal after coarct repair despite the substantial increase in transaortic flow. The valve was left intact.

ASD Repair (Coming Soon)

Next the secundum ASD was closed using a double-thickness bovine pericardial baffle. Although moderate tricuspid regurgitation was initially present, follow-up transthoracic echo showed a decreased RV size and TR was judged trace - mild. The septum repair remained intact. Click here to see an example of congenital sinus venosus ASD repair.